Since the identification of NOTCH3 as a key modular of CADASIL, more inherited causes of CSVD have been identified, including pathogenic variants in genes relating to cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL) and COL4A1/A2-related disorders, amongst others. Here, NOTCH3 is linked to CARASIL.